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JOURNAL ARTICLE
Jing Miao, Filippo Pinto E Vairo, Marie C Hogan, Stephen B Erickson, Mireille El Ters, Andrew J Bentall, Aleksandra Kukla, Eddie L Greene, Loren Herrera Hernandez, Sanjeev Sethi, Konstantinos N Lazaridis, Pavel N Pichurin, Emily Lisi, Carri A Prochnow, Ladan Zand, Fernando C Fervenza
OBJECTIVE: To increase the likelihood of finding a causative genetic variant in patients with a focal segmental glomerulosclerosis (FSGS) lesion, clinical and histologic characteristics were analyzed. PATIENTS AND METHODS: Individuals 18 years and older with an FSGS lesion on kidney biopsy evaluated at Mayo Clinic from November 1, 1999, through October 31, 2019, were divided into 4 groups based on clinical and histologic characteristics: primary FSGS, secondary FSGS with known cause, secondary FSGS without known cause, and undetermined FSGS...
September 2021: Mayo Clinic Proceedings
#2
REVIEW
Kuang-Yu Wei, Martin Gritter, Liffert Vogt, Martin H de Borst, Joris I Rotmans, Ewout J Hoorn
Potassium often has a negative connotation in Nephrology as patients with chronic kidney disease (CKD) are prone to develop hyperkalaemia. Approaches to the management of chronic hyperkalaemia include a low potassium diet or potassium binders. Yet, emerging data indicate that dietary potassium may be beneficial for patients with CKD. Epidemiological studies have shown that a higher urinary potassium excretion (as proxy for higher dietary potassium intake) is associated with lower blood pressure (BP) and lower cardiovascular risk, as well as better kidney outcomes...
December 2020: Clinical Kidney Journal
#3
Martin Bezdíčka, Jan Langer, Jaromír Háček, Jakub Zieg
Dent disease is an X-linked recessive renal tubular disorder characterized by proximal tubule dysfunction. Typical features include low molecular weight proteinuria, hypercalciuria, nephrocalcinosis, nephrolithiasis, rickets, and chronic renal failure. We present a case of a 6-year-old boy with nephrotic proteinuria without hypoalbuminemia or edema. His renal biopsy revealed focal segmental glomerulosclerosis (FSGS), some of the glomeruli were globally sclerotic. Hypercalciuria was present intermittently and urine protein electrophoresis showed low molecular weight protein fraction of 50%...
2020: Frontiers in Pediatrics
#4
REVIEW
Christina L Nelms, Vanessa Shaw, Larry A Greenbaum, Caroline Anderson, An Desloovere, Dieter Haffner, Michiel J S Oosterveld, Fabio Paglialonga, Nonnie Polderman, Leila Qizalbash, Lesley Rees, José Renken-Terhaerdt, Jetta Tuokkola, Johan Vande Walle, Rukshana Shroff, Bradley A Warady
In children with kidney diseases, an assessment of the child's growth and nutritional status is important to guide the dietary prescription. No single metric can comprehensively describe the nutrition status; therefore, a series of indices and tools are required for evaluation. The Pediatric Renal Nutrition Taskforce (PRNT) is an international team of pediatric renal dietitians and pediatric nephrologists who develop clinical practice recommendations (CPRs) for the nutritional management of children with kidney diseases...
April 2021: Pediatric Nephrology
#5
REVIEW
R Geraghty, K Wood, J A Sayer
Calcium oxalate (CaOx) crystal deposition within the tubules is often a perplexing finding on renal biopsy of both native and transplanted kidneys. Understanding the underlying causes may help diagnosis and future management. The most frequent cause of CaOx crystal deposition within the kidney is hyperoxaluria. When this is seen in native kidney biopsy, primary hyperoxaluria must be considered and investigated further with biochemical and genetic tests. Secondary hyperoxaluria, for example due to enteric hyperoxaluria following bariatric surgery, ingested ethylene glycol or vitamin C overdose may also cause CaOx deposition in native kidneys...
October 2020: Urolithiasis
#6
JOURNAL ARTICLE
Martina Živná, Kendrah Kidd, Mohamad Zaidan, Petr Vyleťal, Veronika Barešová, Kateřina Hodaňová, Jana Sovová, Hana Hartmannová, Miroslav Votruba, Helena Trešlová, Ivana Jedličková, Jakub Sikora, Helena Hůlková, Victoria Robins, Aleš Hnízda, Jan Živný, Gregory Papagregoriou, Laurent Mesnard, Bodo B Beck, Andrea Wenzel, Kálmán Tory, Karsten Häeffner, Matthias T F Wolf, Michael E Bleyer, John A Sayer, Albert C M Ong, Lídia Balogh, Anna Jakubowska, Agnieszka Łaszkiewicz, Rhian Clissold, Charles Shaw-Smith, Raj Munshi, Robert M Haws, Claudia Izzi, Irene Capelli, Marisa Santostefano, Claudio Graziano, Francesco Scolari, Amy Sussman, Howard Trachtman, Stephane Decramer, Marie Matignon, Philippe Grimbert, Lawrence R Shoemaker, Christoforos Stavrou, Mayssa Abdelwahed, Neila Belghith, Matthew Sinclair, Kathleen Claes, Tal Kopel, Sharon Moe, Constantinos Deltas, Bertrand Knebelmann, Luca Rampoldi, Stanislav Kmoch, Anthony J Bleyer
There have been few clinical or scientific reports of autosomal dominant tubulointerstitial kidney disease due to REN mutations (ADTKD-REN), limiting characterization. To further study this, we formed an international cohort characterizing 111 individuals from 30 families with both clinical and laboratory findings. Sixty-nine individuals had a REN mutation in the signal peptide region (signal group), 27 in the prosegment (prosegment group), and 15 in the mature renin peptide (mature group). Signal group patients were most severely affected, presenting at a mean age of 19...
December 2020: Kidney International
#7
JOURNAL ARTICLE
Panupong Hansrivijit, Wisit Cheungpasitporn, Charat Thongprayoon, Nasrollah Ghahramani
BACKGROUND: Use of rituximab (RTX) for focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) is widely described in children. Clinical evidence in adults is limited. The objective of this study was to determine the treatment outcomes of RTX in adults with FSGS and MCD. METHODS: Ovid MEDLINE, SCOPUS, and Cochrane Database of Systematic Reviews were searched up to September 2019. Out of 699 studies, we included 16 studies describing the treatment outcomes of rituximab in adult patients with FSGS or MCD...
April 15, 2020: BMC Nephrology
#8
REVIEW
Wooin Ahn, Andrew S Bomback
Podocyte injury is the initiating step in the pathway toward clinically evident forms of nephrotic syndrome known as podocytopathies, represented as either minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS). There are hallmark differences in the histologic appearances of MCD and FSGS, which in turn represent distinct pathogenic models after initial podocyte injury (eg, no change in podocyte number in MCD vs podocyte detachment and death in FSGS). However, MCD and FSGS also share a number of common causes, supporting the theory that these diseases lie along a shared podocytopathy spectrum...
June 2020: American Journal of Kidney Diseases
#9
JOURNAL ARTICLE
Ulf Schönermarck, Wolfgang Ries, Bernd Schröppel, Lars Pape, Malgorzata Dunaj-Kazmierowska, Volker Burst, Steffen Mitzner, Nadezda Basara, Michael Starck, Daniel Schmidbauer, Alexander Mellmann, Rita Dittmer, Michael Jeglitsch, Christian S Haas
BACKGROUND: Data are lacking on the relative incidence of thrombotic thrombocytopenic purpura (TTP), haemolytic uraemic syndrome (HUS) caused by Shiga toxin-producing Escherichia coli (STEC) and atypical HUS (aHUS) in patients presenting with thrombotic microangiopathies (TMAs). METHODS: This was a prospective, cross-sectional, multicentre and non-interventional epidemiological study. Patients fulfilling criteria for TMAs (platelet consumption, microangiopathic haemolytic anaemia and organ dysfunction) were included in the study...
April 2020: Clinical Kidney Journal
#10
REVIEW
Priyanka Khandelwal, Jasintha Sabanadesan, Aditi Sinha, Pankaj Hari, Arvind Bagga
Identification of a monogenic etiology is possible in a proportion of patients with childhood-onset nephrolithiasis or nephrocalcinosis. Bartter syndrome (BS), a hereditary tubulopathy characterized by polyuria, hypokalemic alkalosis and growth retardation that rarely presents with isolated nephrocalcinosis. Patients with defect in renal outer medullary potassium channel, encoded by the KCNJ1 gene causing BS type 2, typically present during the neonatal period. We describe a 14-year-old girl with mild late-onset BS type 2 with reported pathogenic compound heterozygous variations in exon 2 of KCNJ1 (c...
August 2020: CEN Case Reports
#11
JOURNAL ARTICLE
Mohan Shenoy, Rachel Lennon, Nick Plant, Dean Wallace, Amrit Kaur
BACKGROUND: Children with non-genetic steroid-resistant nephrotic syndrome (SRNS) are at high risk of disease recurrence (DR) and graft loss following renal transplant (RT). Although pre-emptive plasma exchange (PE) and rituximab have been suggested to prevent DR, there is insufficient published data to support this practice. The aim is to study the role of pre-emptive PE and rituximab in the prevention of DR in children with non-genetic SRNS undergoing living donor (LD) RT. METHODS: Prospective single-centre study of four consecutive children (age 6-17 years) with non-genetic SRNS (including two with previous graft loss due to DR) who underwent LD RT between July 2014 and September 2016...
June 2020: Pediatric Nephrology
#12
JOURNAL ARTICLE
Aureliusz Kolonko, Natalia Słabiak-Błaż, Henryk Karkoszka, Andrzej Więcek, Grzegorz Piecha
Proteasome inhibitor bortezomib has been used in the treatment of refractory cases of acute and chronic antibody-mediated rejection (AMR) in kidney transplant recipients. However, its efficacy and safety as a primary treatment for early AMR has been scarcely investigated. We herein present our preliminary experience with bortezomib- and plasmapheresis-based primary treatment for early AMR. Thirteen patients transplanted between October 2015 and September 2019 were treated (starting at median 19th post-transplant day) with bortezomib/plasmapheresis protocol for early biopsy-proven AMR...
February 15, 2020: Journal of Clinical Medicine
#13
REVIEW
Rosanna Coppo
IgA nephropathy (IgAN) in children is no longer considered a rare and benign disease but a nephritis with different presentations and various outcomes. The decision to initiate a treatment and the therapeutic choice depend on the individual risk of progression. The Kidney Disease: Improving Global Outcomes (KDIGO) clinical guidelines in 2012 considered that the risk factors for progression of IgAN were similar in both children and adults and suggested in some conditions to follow the adult schedules. In 2017 a KDIGO Controversies Conference on management and treatment of glomerular diseases decided not to include an update in children with IgAN since the level of evidence of treatments in children was too scarce...
March 2021: Pediatric Nephrology
#14
RANDOMIZED CONTROLLED TRIAL
Julie Refardt, Cornelia Imber, Clara O Sailer, Nica Jeanloz, Laura Potasso, Alexander Kutz, Andrea Widmer, Sandrine A Urwyler, Fahim Ebrahimi, Deborah R Vogt, Bettina Winzeler, Mirjam Christ-Crain
BACKGROUND: Treatment options to address the hyponatremia induced by the syndrome of inappropriate antidiuresis (SIAD) are inadequate. The sodium glucose cotransporter 2 (SGLT2) inhibitor empagliflozin promotes osmotic diuresis via urinary glucose excretion and therefore, might offer a novel treatment option for SIAD. METHODS: In this double-blind, randomized trial, we recruited 88 hospitalized patients with SIAD-induced hyponatremia <130 mmol/L at the University Hospital Basel from September 2016 until January 2019 and assigned patients to receive, in addition to standard fluid restriction of <1000 ml/24 h, a once-daily dose of oral empagliflozin or placebo for 4 days...
March 2020: Journal of the American Society of Nephrology: JASN
#15
RANDOMIZED CONTROLLED TRIAL
Dominique M Bovée, Wesley J Visser, Igor Middel, Anneke De Mik-van Egmond, Rick Greupink, Rosalinde Masereeuw, Frans G M Russel, A H Jan Danser, Robert Zietse, Ewout J Hoorn
BACKGROUND: Distal diuretics are considered less effective than loop diuretics in CKD. However, data to support this perception are limited. METHODS: To investigate whether distal diuretics are noninferior to dietary sodium restriction in reducing BP in patients with CKD stage G3 or G4 and hypertension, we conducted a 6-week, randomized, open-label crossover trial comparing amiloride/hydrochlorothiazide (5 mg/50 mg daily) with dietary sodium restriction (60 mmol per day)...
March 2020: Journal of the American Society of Nephrology: JASN
#16
REVIEW
Fay J Dickson, John A Sayer
The abnormal deposition of calcium within renal parenchyma, termed nephrocalcinosis, frequently occurs as a result of impaired renal calcium handling. It is closely associated with renal stone formation (nephrolithiasis) as elevated urinary calcium levels (hypercalciuria) are a key common pathological feature underlying these clinical presentations. Although monogenic causes of nephrocalcinosis and nephrolithiasis are rare, they account for a significant disease burden with many patients developing chronic or end-stage renal disease...
January 6, 2020: International Journal of Molecular Sciences
#17
JOURNAL ARTICLE
Maxime Coutrot, Francois Dépret, Matthieu Legrand
Hyperkalemia is a common electrolyte disorder that may be rapidly life-threatening because of its cardiac toxicity. Hyperkalemia risk factors are numerous and often combined in the same patient. Most of the strategies to control serum potassium level in the short term have been used for decades. However, evidence for their efficacy and safety remains low. Treatment of hyperkalemia remains challenging, poorly codified, with a risk of overtreatment, including short-term side effects, and with the priority of avoiding unnecessary hospital stays or chronic medication changes...
December 1, 2019: Nephrology, Dialysis, Transplantation
#18
REVIEW
Bernhard Banas, Bernhard K Krämer, Bernd Krüger, Nassim Kamar, Nasrullah Undre
Tacrolimus has significantly improved outcomes for kidney transplant patients and remains the cornerstone of immunosuppressive therapy. While improvements in short-term outcomes in transplantation have been achieved in recent years, maintaining long-term graft survival remains a challenge in kidney transplantation. Minimizing risk factors for poor long-term kidney graft function and survival, and modifying tacrolimus regimens in the early and maintenance phases post-transplantation are essential to maintain long-term kidney transplant outcomes...
January 2020: Transplantation Proceedings
#19
JOURNAL ARTICLE
Yohan Bignon, Laurent Pinelli, Nadia Frachon, Olivier Lahuna, Lucile Figueres, Pascal Houillier, Stéphane Lourdel, Jacques Teulon, Marc Paulais
The kidneys excrete the daily acid load mainly by generating and excreting ammonia but the underlying molecular mechanisms are not fully understood. Here we evaluated the role of the inwardly rectifying potassium channel subunit Kir4.2 (Kcnj15 gene product) in this process. In mice, Kir4.2 was present exclusively at the basolateral membrane of proximal tubular cells and disruption of Kcnj15 caused a hyperchloremic metabolic acidosis associated with a reduced threshold for bicarbonate in the absence of a generalized proximal tubule dysfunction...
February 2020: Kidney International
#20
JOURNAL ARTICLE
Guglielmo Schiano, Bob Glaudemans, Eric Olinger, Nadine Goelz, Michael Müller, Dominique Loffing-Cueni, Georges Deschenes, Johannes Loffing, Olivier Devuyst
Uromodulin, the most abundant protein in normal urine, is produced by cells lining the thick ascending limb (TAL) of the loop of Henle. Uromodulin regulates the activity of the potassium channel ROMK in TAL cells. Common variants in KCNJ1, the gene encoding ROMK, are associated with urinary levels of uromodulin in population studies. Here, we investigated the functional link between ROMK and uromodulin in Kcnj1 knock-out mouse models, in primary cultures of mouse TAL (mTAL) cells, and in patients with Bartter syndrome due to KCNJ1 mutations...
December 20, 2019: Scientific Reports
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